Pediatric Examination and Board Review (46 page)

A newborn girl named Anita presents with a lumbar meningomyelocele, noted in the delivery room of a community hospital. Mom is a 29-year-old woman with two normal children. The pregnancy was uneventful. Because of failure of labor to progress after 24 hours, a cesarean delivery was performed using general anesthesia. You are summoned by your emergency beeper and arrive 15 minutes later to the hospital obstetric operative delivery room. The father is in the delivery room looking pale and tearful.

On physical examination, Anita is 6 pounds, robust and has a vigorous cry, a good urine stream, spontaneous knee flexion and extension, and club feet. The delivery room nurses have applied a sterile dressing to Anita’s back.

SELECT THE ONE BEST ANSWER

1.
How will you communicate your initial concerns to Anita’s father?

(A) I am concerned that there might be something seriously wrong with Anita’s brain

(B) if Anita’s mother had not run out of prenatal vitamins, this would not have happened

(C) Anita seems to have had a disorder impacting on her spinal cord; even though it looks very abnormal, specialists in pediatric neurosurgery can assist us in management

(D) we should make Anita comfortable but not do anything heroic

(E) you do not communicate any initial concerns; allow the neurosurgeon to evaluate and talk to Anita’s father

2.
Known etiologies or associations of neural tube disorders include all of the following except

(A) taking prenatal vitamins before conception

(B) chromosomal disorders

(C) maternal anticonvulsants

(D) first-trimester hyperthermia

(E) multiple malformation syndromes

3.
You inform both parents together of your concerns. Which is the most appropriate statement?

(A) Anita has spina bifida and will be severely handicapped

(B) I don’t know how to fix this

(C) the mother might have drunk too much alcohol in early pregnancy

(D) the mother must have had unrecognized diabetes in pregnancy

(E) we are going to transfer Anita to a tertiary care center where specialists will help

4.
All of the following pertinent initial findings are reassuring except

(A) the anterior fontanelle is flat

(B) nystagmus is present

(C) there is a vigorous Moro reflex

(D) there is decreased movement of the feet

(E) B and D

5.
Associated findings or complications of spina bifida include all of the following except

(A) hydrocephalus

(B) neurogenic bowel and bladder

(C) severe mental retardation

(D) kyphoscoliosis

(E) difficulty with lower extremity sensation

6.
At age 4
¹
/
₂
, Anita arrives in your office with an aluminum lightweight wheelchair, which she expertly self-propels down the hall. She locks her brakes and, with lightweight braces and crutches, walks to a small table. Which of the following is true?

(A) Anita will not need a wheelchair as a teenager

(B) Anita will learn to do her own bladder catheterization

(C) Anita has no risk for a shunt malfunction

(D) Anita is severely retarded

(E) Anita does not need to take special latex precautions if she has not had a latex allergic reaction

7.
In kindergarten, Anita is able to walk with braces and a walker. She sings songs, speaks clearly in 5- or 6-word sentences, and knows colors, the alphabet, and counting. She is able to draw a circle and a cross but not a triangle. She is left-handed. She can construct a 3-piece bridge and a 5-piece gate with blocks. Her drawing of a person consists of a head with two stick legs. She is not yet toilet trained. Anita’s developmental assessment is

(A) severe mental retardation

(B) severe reading disability

(C) mild perceptual delays

(D) pathologic left-handedness

(E) poor self-image

8.
All of the following are signs of developmental readiness for daytime toilet training except

(A) can pull pants off

(B) shows awareness of wetness

(C) when the parent sits the child on the toilet every 2 hours, will urinate 25% or more of the time

(D) understands cause and effect

(E) can follow 2-step commands and speaks in 2-word phrases

9.
All of the following statements about toileting are true except

(A) stools should be treated with disgust

(B) the most important outcome is the child’s sense of self-esteem and task-mastery

(C) it is wrong to fight, punish, shame, or nag

(D) girls achieve toileting earlier than boys

(E) imitating parents and siblings is helpful

10.
Which of the following statements is true?

(A) all children with Down syndrome require diapers in kindergarten

(B) children with spina bifida cannot learn independent catheterization until adolescence

(C) nocturnal enuresis occurs in 25% of 4-year-olds

(D) the lowest rate of success for nocturnal enuresis is with alarms

(E) enuresis does not run in families

11.
In preadolescents with chronic illness, indicators of low self-esteem include all of the following except

(A) completing a board game even if one is losing

(B) not trying something for fear of failure

(C) acting silly to minimize feeling like a failure

(D) saying, “I can’t do anything right.”

(E) all of the above are indicators of low self-esteem

ANSWERS

1.
(C)
Anita has a lower lumbar meningomyelocele. Initial management includes keeping the wound sterile and closure by an experienced pediatric neurosurgeon.

Recent advances include the recognition that folate decreases the primary risk of meningomyelocele and recurrence risk in subsequent pregnancies. The goal of a pediatrician is to be supportive of the family while arranging for supportive consultations. Too many fears are raised by not systematically describing spina bifida, its developmental impact, and its management. It is inappropriate to blame the mother for spina bifida.

2.
(A)
Prenatal vitamins do not cause spina bifida but are preventive for the disorder in a substantial number of individuals. Maternal anticonvulsants (eg, valproate), chromosomal disorders, first-trimester maternal hyperthermia, and Meckel syndrome (cleft, polydactyly, cystic liver and kidneys) are associated with spina bifida.

3.
(E)
90% of children with meningomyelocele have hydrocephalus secondary to Arnold Chiari malformations. Hydrocephalus is currently managed with ventriculoperitoneal shunts.

4.
(E)
Although a vigorous Moro reflex and a flat anterior fontanelle are reassuring, nystagmus and decreased pedal movement reflect brainstem and spinal cord dysfunction.

5.
(C)
Unless increased intracranial pressure is present or ventriculitis ensues, cognitive impairments are subtle and mild (ie, learning disabilities, slow learner, mild cognitive impairment).

6.
(B)
Anita is already demonstrating the problemsolving skills of alternative mobility. With training and support, she will learn clean intermittent catheterization techniques. Anita will need a wheelchair as a teenager. She is always at risk for a shunt malfunction. She is not severely retarded. More than a third of children with spina bifida have latex sensitivity. There have been reports of latex anaphylaxis. A latex-free environment is currently recommended for children with spina bifida.

7.
(C)
Anita has mild perceptual delays reflected in her difficulty drawing a triangle and not completing more details during her attempt to draw a person.

8.
(C)
Although having some successes on the toilet is comforting, there should be more than 25% success when put on the toilet every 2 hours.

9.
(A)
Given children’s imaginations and easily provoked fears, stools should not be demeaned but treated in a matter-of-fact manner.

10.
(C)
Nocturnal enuresis occurs in 25% of 4-yearolds, 10% of 8-year-olds, and 2% of 13-year-olds.

11.
(A)
Children with low self-esteem, like Anita, often avoid trying something new, act silly, and are selfderogatory. Finishing a game when one is losing reflects a maturity of learning from one’s mistakes.

S
UGGESTED
R
EADING

Jacobs RA.
Myelodysplasia (spina bifida-myelomeningocele)
. In: Wolraich ML, ed.
Disorders of Development and Learning.
3rd ed. Hamilton, Ontario, Canada: BC Decker; 2003:137-174.

Jacobs RA. Spina bifida. In: Rubin IL, Crocker AC, eds.
Medical
Care for Children and Adults with Developmental Disabilities.
2nd ed. Baltimore, MD: Paul H. Brookes; 2006:139-152.

Sandler A.
Living with Spina Bifida: A Guide for Families and
Professionals
. Chapel Hill, NC: University of North Carolina Press; 1997.

CASE 26: A 3-YEAR-OLD WITH SPEECH DELAY